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ORGAN ORGAN
DONATION DONATION

Pediatric Liver Transplantation: disease. For young adults >20

years post-transplant, health-
A path to life beyond liver disease related quality of life is similar
to the general population of
young adults in the U.S. across
By Danielle Fritze, MD, and Francisco G. Cigarroa, MD most domains. Outcomes
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also continue to improve with
W hile end stage liver disease prompting transplantation is most thrive after transplant. Most children will benefit from appropriate vac- time, with patient and graft
survival for pediatric liver re-
common later in life, infants, children and adolescents are
cination and younger children with chronic liver disease often require
also impacted. Each year in the U.S., around 500 children
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in the U.S. since 2010.
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a new liver. The timing of transplant evaluation is determined by the
receive a new liver, yet more than 1,000 remain on the waitlist. Unfor- intensive nutritional interventions to promote growth while awaiting cipients on an upward trend
tunately, waitlist mortality remains a significant problem, and the severity and trajectory of a child’s liver disease, but early referral is ben- Pediatric liver transplanta-
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youngest patients are at the greatest risk. Living donor liver transplant eficial. For children with acute liver failure, who are at risk for imme- tion is a technically complex
(LDLT) is an important means of access to transplantation that is not diate progression to cerebral edema and death, full evaluation and operation, and the care of pe-
dependent upon a child’s priority on the waitlist. While pediatric liver listing can occur in a matter of hours. diatric liver candidates and
recipients may be extremely ill from their liver disease prior to transplant, In the United States, the allocation of deceased donor livers for trans- liver recipients requires a
outcomes remain excellent with 1-, 5- and 10-year post-transplant sur- plant is determined primarily by acuity, secondarily factoring in a re- highly specialized and dedi-
vival of 94 percent, 90 percent and 88 percent, respectively. cipient’s waiting time and the distance between the organ and recipient. cated team of transplant pro-
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The indications for liver transplant in children represent a broad Livers are thus allocated first to those with the highest risk of mortality, fessionals. Living donor liver
array of conditions, with approximately half involving cirrhosis. Biliary typically patients in fulminant hepatic failure, and then to those who liver recipients. Critically, the availability of a suitable living donor al- transplant is a key means of access to timely transplantation, particularly
atresia is the most common indication, comprising 40 percent of pedi- are more critically ill with chronic liver disease. There is not a separate lows a child to receive a liver transplant in the timeframe that is most for infants and children, and a pathway to life beyond liver disease.
atric liver transplants. Auto-immune hepatitis, Primary Sclerosing waitlist for children, but children do receive priority for livers from pe- optimal for their health, often before they become critically ill. For chil- Additional information about pediatric liver transplantation and liv-
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Cholangitis, Progressive Familial Intrahepatic Cholestasis, and severe diatric and adolescent donors. dren in fulminant hepatic failure who may not receive a suitable de- ing liver donation can be found at our website: https://www.universi-
Cholestasis related to Total Parenteral Nutrition are other causes of cir- On a population scale, this liver allocation system is designed to save ceased donor liver offer in time, living donor liver transplant may tyhealth.com/services/transplant-care.
rhosis and transplantation in children. Unlike in adults, nearly half of lives and avoid waitlist mortality by directing livers first to patients in literally be the difference between life and death. For families, the ex-
pediatric liver transplants are performed in children who do not have the most urgent need. For an individual patient, the unfortunate reality perience of living donor liver transplant has many advantages. Rather References:
cirrhosis. Several primary hepatic neoplasms, most notably unresectable of this system is that the child may become critically ill before they rise than receiving an unexpected phone call and having to rush to the hos- 1. Scientific Registry of Transplant Recipients Annual Report 2021
hepatoblastoma (without evident extrahepatic disease), have high cure high enough on the waitlist to receive a liver transplant. In 2021, 5.4 pital when a donor liver becomes available, families now have the op- 2. https://www.srtr.org/transplant-centers/interactive-report?cen-
rates with liver transplantation. A number of metabolic disorders can percent of children on the waitlist either died waiting for a transplant, portunity to mark the date of their child’s transplant on the calendar ter=TXUC&type=TX1&organ=li
also be effectively treated with liver transplantation. In ornithine tran- or were removed from the list when they became too sick to undergo weeks in advance. This allows time to make preparations for additional 3. Mohammad S., et al. Health status in young adults 2 decades after
scarbamylase deficiency and maple syrup urine disease, for example, liver transplantation. Waitlist mortality is highest in children less than support, and arrangements for work absences, childcare, etc. Donors pediatric liver transplantation. AJT 2012 Jun; 12(6): 1486-1495.
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liver transplantation effectively functions as a form of gene therapy — a year old. This cohort of the youngest liver patients has particularly report that the ability to intervene definitively to save the life of a child
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the missing enzyme is produced by the healthy transplanted liver to re- limited access to transplantation due to their small abdominal domain is extremely rewarding. Danielle Fritze, MD, completed her medical degree and sub-
store the affected metabolic pathway. Finally, acute liver failure accounts (limiting the size of the liver that may be transplanted), and the paucity Our extraordinary Champion for Life program, recipient of the sequent surgical training at the University of Michigan. Following
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for approximately 10 percent of pediatric liver transplants. Many of of pediatric donors. For many children, the answer is transplantation 2023 Pinnacle Award from Donate Life America, helps each family to general surgery residency, she pursued additional fellowship train-
these cases are triggered by a non-hepatitis viral illness such as aden- with a portion of a larger liver, termed a split liver graft, from either a identify an individual who can help to spread the word about the child’s ing in transplantation and hepatobiliary surgery before joining the faculty
ovirus or enterovirus in a child with a previously healthy liver. Hepatitis deceased or living donor. In the past decade, living donor liver trans- need for a living donor, termed a “Living Donor Champion.” The at UT Health San Antonio. Dr. Fritze specializes in liver and kidney
B, toxic ingestions including acetaminophen, autoimmune and meta- plantation has been established as the modality of choice for most chil- Champion for Life program then educates the donor champion about transplantation as well as surgery of the liver, bile duct and pancreas. She
bolic conditions can also lead to acute liver failure. While the immedi- dren, with fewer graft losses and improved overall survival compared liver disease, transplantation and living donation, and equips the donor has a particular interest in pediatric transplantation and was named the
ate mortality of fulminant hepatic failure is high, children who receive to deceased donor liver transplant (5-year survival: 91 percent vs. 85 champion to effectively share the patient’s story. Donors may be related, McCombs Director of Pediatric Transplantation. Dr. Fritze is a member
a timely liver transplant have an excellent prognosis. Prompt referral to percent). unrelated or even anonymous. In the past three years, altruistic non- of the Bexar County Medical Society.
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a pediatric liver transplant center is thus crucial. In our practice, outstanding access to living donor liver transplanta- directed donors — individuals who volunteered to donate part of their
A child’s candidacy for liver transplantation is determined by a multi- tion has been transformational. Overall transplant rates have increased, liver to anyone in need — have saved the lives of seven children through Francisco G. Cigarroa, MD, is the Director of the Malu’ and
disciplinary team who comprehensively reviews each child’s individual currently more than twice the expected rate and more than five times living donor liver transplantation at our transplant center. Carlos Alvarez Center for Transplantation, Hepatobiliary Sur-
case — primary disease process, comorbidities, hepatic anatomy and the national rate. Median wait time is significantly shorter for those For those children fortunate enough to receive a transplant, the out- gery and Innovation. He specializes in pediatric and adult kidney
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social support. Few contraindications are absolute; barriers are identi- children receiving a living donor liver transplant, but graft and patient look is bright. Survival curves flatten after the first-year post- and liver transplantation. He holds the Alvarez Distinguished University
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fied and addressed prior to transplant to pave the way for the child to survival have remained excellent for both living and deceased donor transplant, with improved quality of life compared to chronic liver Chair and the Ashbel Smith Professorship in Surgery.

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